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Intrathoracic lymphangioma.

L R Brown, H M Reiman, E C Rosenow

    Mayo Clinic Proceedings
    |November 1, 1986
    PubMed
    Summary
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    Intrathoracic lymphangiomas, benign tumors, often recur due to their infiltrative nature, complicating surgical removal. Imaging like CT scans aids in diagnosis and assessing the extent of these mediastinal lymphatic malformations.

    Area of Science:

    • Thoracic surgery
    • Diagnostic imaging
    • Pediatric oncology

    Background:

    • Mediastinal lymphangiomas are rare congenital malformations.
    • Understanding their varied presentations and locations is crucial for diagnosis and management.

    Observation:

    • Of 14 intrathoracic lymphangioma cases, distribution varied: 4 anterior, 5 superior, and 4 posterior mediastinum.
    • One diffuse case presented with Gorham's disease (disappearing bone disease).
    • Anterior lesions occurred in adults, superior lesions in children (often extensions of cervical cystic hygromas), and posterior lesions showed varied presentations.

    Findings:

    • Anterior mediastinal lymphangiomas were indistinguishable from other lesions on plain radiography.
    • Computed tomography (CT) effectively identified cervical extensions and cystic nature of superior lesions.

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  • Posterior lesions included isolated cases and extensive lymphangiomatosis with bone involvement and subdiaphragmatic extension.
  • Chylothorax complicated two posterior cases post-surgery.
  • CT and lymphangiography were vital for determining disease extent and nature.

    • Implications:

    • The infiltrative nature of lymphangiomas complicates complete resection, leading to common postoperative recurrence.
    • Accurate staging with imaging is essential for surgical planning and managing complications like chylothorax.
    • Multidisciplinary approaches may be necessary for extensive or recurrent cases, especially those associated with syndromes like Gorham's disease.